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Dermatopathology
The Dermatopathologic Manifestations of Systemic Disease:
Novel Considerations Regarding Cutaneous Lymphoid Proliferations
Sunday, February 27, 2005 - 7:30 PM
Rivercenter Salon E
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Moderator:
A. Neil Crowson
Regional Medical Lab
Tulsa, OK
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Click on each slide thumbnail image for an enlarged view
Submitted by:
A. Neil Crowson
Regional Medical Lab
Tulsa, OK
A 74 year old woman developed a psoriasiform, papular and plaque-like eruption of the back, arms,
legs, palms and soles whilst on Cozaar (losarten – a potent angiotensin-type I receptor blocker),
atenolol (a beta-blocker), the diuretic hydrochlorothiazide and verapamil (a calcium channel blocker).
There was no involvement of facial skin and no lymphadenopathy was detectable. Apart from severe
arterial hypertension, an examination and review by an internist showed no systemic disease. An initial
skin biopsy in December 2000 showed a superficially disposed atypical small lymphoid infiltrate with
coarse sclerosis of the collagen table, in concert with an epidermotropic atypical lymphoid infiltrate
suggesting large plaque parapsoriasis in evolution to mycosis fungoides (Figs 1A, 1B and 1C). Flow
cytometry of peripheral blood showed changes consistent with a reactive process by virtue of a Th/s
ration of 2.6:1 with normal expression of CD2,3,5 and 7 with no aberrant loss of surface antigen
expression; no Sezary cells were detectable. After subsequent enquiries concerning the drug history, the
patient was referred to a dermatologist with a specific interest and training in pharmacotherapeutics,
and a second biopsy was performed in January 2001 followed by modulation of the drug regime in concert
with topical steroid therapy (0.1% triamcinolone). As the last agent added to the patient's drug regime was the
cozaar, that agent was discontinued first, in concert with the thiazide. The second biopsy pair showed
similar features to the first, but with important distinctions. First, the intensity of the infiltrate
was much reduced, as was the degree of epidermotropism. Secondly, the involvement of the epidermis
was patchy as opposed to continuous (Figs 2A and 2B). At follow up 2 months later the rash was slightly
improved and she commenced tanning bed therapy for 3 minutes 3-4 times week. At follow up 1 month later
new lesions were still developing and the atenolol was then discontinued. Three weeks later the eruption
was markedly improved with a reduction of the involved surface area by 80%. She then restarted cozaar
under the guidance of her internist, with an immediate and dramatic flare of the eruption on examination
in June 2001. The cozaar was again discontinued and by July the rash was largely resolved. In September
the verapamil was also discontinued. By October 2001 all that remained of the rash were two small
eczematous patches on the back. Clonidine was substituted for anti-hypertensive therapy and atenolol was
re-introduced. The tanning bed was tapered. The conclusion was that cozaar and verapamil were
synergistic in provocation of the eruption.
Her hypertension was unstable and 18 months later an alternate calcium channel blocker and beta
blocker (Toprol and Norvasc respectively) were substituted with flaring of the eruption. A rebiopsy at
that time (January 2003) showed changes held to be compatible with a lymphomatoid drug eruption (Figs 3A
and 3B). In particular, there was directed migration of small lymphoid forms into areas of maximum
antigenic processing, namely, acrosyringia and hair follicles, with a significant reduction in the
intensity of the infiltrate. The calcium channel blocker was discontinued, methyldopa was substituted,
and the rash resolved. She remains in dermatologic remission, with no evidence of a rash on the
previously involved trunk at the last examination in October of 2004 (Fig 4).
Submitted by:
A. Neil Crowson
Regional Medical Lab
Tulsa, OK
A 68-year-old male presented with a solitary fleshy 1.4 x 0.9 cm diameter lesion on his back. A shave
biopsy was interpreted as a lichenoid tissue reaction with epidermal hyperplasia; no atypical lymphoid
cells were seen in the infiltrate. Over the next eight weeks, the lesion grew to a 2.8 x 2.5 cm
tumefactive nodule and a second biopsy was obtained.
Submitted by:
Lorenzo Cerroni
Medical University of Graz
Graz, Austria
A 86-year-old woman with a large erythematous tumor on her left lower leg (Fig 1). The tumor was
slowly growing for the last few months. The surrounding skin was normal. Complete staging
investigations, including bone marrow biopsy, were negative. The patient had neither paraproteinemia nor
Bence-Jones proteins in the urine. A biopsy was performed under local anesthesia.
Submitted by:
Lorenzo Cerroni
Medical University of Graz
Graz, Austria
A 15-year-old boy presented with sudden onset of eroded papules and small vesicles on the glans penis
and prepuce. A biopsy was taken under local anesthesia. Subsequent staging investigations at
presentation were negative.
Submitted by:
Cynthia M. Magro
Ohio State University
Columbus, OH
A 36-year-old female status post living related donor renal transplant in 1991 developed redness,
swelling and warmth in her right lower extremity from the mid-calf down in September 2001. Her primary
care physician treated her with antibiotics for a presumptive diagnosis of cellulitis without
improvement. In March 2002, an incisional biopsy was performed.
The EBER RT-IS-PCR assay showed numerous cells demonstrating dominant nuclear expression; the vTK
assay was negative. HHV8 RT-IS-PCR revealed positive co-expression of the virus in the tumor cells. The
oligoprobe assay showed λ light chain restriction. Subsequent studies showed monoclonal IgG λ
paraproteinemia. Imaging studies demonstrated a soft tissue mass associated with her transplanted
kidney, but a biopsy was never obtained.
At the time of the diagnosis of plasmablastic lymphoma, she had been on immunosuppressive therapy for
11 years with cyclosporin, imuran and prednisone. She was treated with a decrease in her
immunosuppressive regimen and was started on intravenous acyclovir. This resulted in normalization of
the PET scan; however, her cutaneous disease continued to progress. Surgical resection of the mass
revealed a diffuse large B cell lymphoma with λ light chain restriction; many of the cells showed
EBER-1 and EBER-2 expression. Following resection of the tumor mass, she was treated with local external
beam radiation with resolution of the skin lesions. She has had no cutaneous recurrences to date, but
she has a persistent, albeit quantitatively decreased, IgG lambda monoclonal gammopathy and persistence
of the mass within the transplanted kidney.
Submitted by:
Cynthia M. Magro
Ohio State University
Columbus, OH
The patient was a 65 year old woman who first presented in 2001 with a left posterior thigh mass. She
was asymptomatic at this time. A biopsy was performed and she underwent a complete hematologic
assessment which did not reveal disseminated extracutaneous disease. She then she was treated with
systemic chemotherapy
The patient then developed a recurrence at the same site in 2003. The tumor was morphologically and
phenotypically identical to the earlier tumor although the cells were smaller and exhibited less atypism.
Approximately 6 months later another recurrence developed which was now compatible with a large cell T
cell lymphoma still manifesting CD20 positivity, weak CD10 expression and prominent CD8 staining. Most
recently in November of 2004 she developed another recurrence compatible with a diffuse large cell T cell
lymphoma exhibiting a CD8 phenotype with co-expression of CD20. As well molecular studies revealed a
pure population of T cells; in addition there was evidence of B cell clonality with two defined molecular
peaks constituting essentially 100% of the infiltrate. She is currently alive and well with persistent
cutaneous disease although in the absence of any known extracutaneous dissemination.
Submitted by:
Sabine Kohler
Stanford University
Palo Alto, CA
A 79 year old man with progressive erythematous tumors on left lower leg. Also has several papules on
right lower leg.
Submitted by:
Sabine Kohler
Stanford University
Palo Alto, CA
A 62 y.o. man with diagnosis of lupus profundus and 7 year history of recurrent subcutaneous nodules.
Now presents with fevers, chills and fatigue.
Submitted by:
Elaine S. Jaffe
National Cancer Institute
NIH, Bethesda, MD
A 7 year old girl presented with an enlarging lesion of the right forehead, over a period of 8
months. A bone marrow biopsy was reported as negative. The skin lesion was biopsied.
Submitted by:
Elaine S. Jaffe
National Cancer Institute
NIH, Bethesda, MD
A 51 year old female had a history of systemic mastocytosis associated with a myelodysplastic
syndrome. She underwent an HLA-matched peripheral blood stem cell transplant. Approximately 3 years
later she developed a scalp mass. A biopsy was obtained.
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